Saline at lower tonicity in cystic fibrosis (SALTI-CF) trial comparing 0.9% 3% 6% nebulised saline.

Background: In people with cystic fibrosis (CF), regular nebulisation of 6% or 7% saline improves lung function; however, these concentrations are not always tolerable. Clinically, some CF patients report using lower concentrations of saline to improve tolerability, yet the effects of lower concentrations are unknown. This study therefore aimed to evaluate the relative effectiveness and tolerability of 0.

Effect of -Acetylcysteine in Combination with Antibiotics on the Biofilms of Three Cystic Fibrosis Pathogens of Emerging Importance.

Cystic fibrosis (CF) is a genetic disorder causing dysfunctional ion transport resulting in accumulation of viscous mucus that fosters chronic bacterial biofilm-associated infection in the airways. and are increasingly prevalent CF pathogens and while is slowly decreasing; all are complicated by multidrug resistance that is enhanced by biofilm formation. This study investigates potential synergy between the antibiotics ciprofloxacin (0.

Elicitation interview study to identify salient beliefs about exercise participation in adults with cystic fibrosis.

Objectives: Conduct an elicitation study, using the Theory of Planned Behaviour framework, to identify salient beliefs about exercise participation in adults with cystic fibrosis (CF). Specifically, identify attitudes on advantages and disadvantages of exercise (behavioural beliefs); individuals and groups who apply social pressure to exercise (normative beliefs); and perceived control over facilitators and barriers to exercise (control beliefs) for adults with CF.

Design: Qualitative interviews using open-ended, structured questions.

Management of Australian Adults with Bronchiectasis in Tertiary Care: Evidence-Based or Access-Driven?

Purpose: Australian data regarding the management of patients with bronchiectasis is scarce. We sought to compare the management of adults with bronchiectasis attending tertiary Australian centres with recent national and international guidelines.

Methods: The Australian Bronchiectasis Registry is a centralised database of patients with radiologically confirmed bronchiectasis unrelated to cystic fibrosis recruited from 14 tertiary Australian hospitals.

Australian adults with bronchiectasis: The first report from the Australian Bronchiectasis Registry.

Background: /objective: There are no large, multi-centre studies of Australians with bronchiectasis. The Australian Bronchiectasis Registry (ABR) was established in 2015 to create a longitudinal research platform. We aimed to describe the baseline characteristics of adult ABR participants and assess the impact of disease severity and exacerbation phenotype on quality of life (QoL).

Side lying during nebulisation can significantly improve apical deposition in healthy adults and adults with mild cystic fibrosis lung disease: a randomised crossover trial.

Background: In people with and without Cystic Fibrosis (CF), does side lying during nebulisation change: the proportion of the dose loaded in the nebuliser that is deposited in the lungs; the uniformity of deposition throughout the lungs; or the apical drug density as a percentage of the drug density in the remaining lung? Do these effects differ depending on the degree of lung disease present?

Methods: A randomised crossover trial with concealed allocation, intention-to-treat analysis and blinded assessors, involving 39 adults: 13 healthy, 13 with mild CF lung disease (FEV > 80%pred), and 13 with more advanced CF lung disease (FEV < 80%pred). In random order, 4 mL of nebulised radioaerosol was inhaled in upright sitting and in alternate right and left side lying at 2-min intervals, for 20 min.

Results: Compared to sitting upright, lung deposition and the uniformity of deposition were not significantly altered by side lying in any of the three groups.

Non-invasive ventilation versus oxygen therapy in cystic fibrosis: A 12-month randomized trial.

Background And Objective: No published studies have examined the long-term effects of non-invasive ventilation (NIV) in cystic fibrosis (CF). Our primary aim was to determine if adults with CF and sleep desaturation were less likely to develop hypercapnia with NIV ± O compared to low-flow oxygen therapy (LFO ) or meet the criteria for failure of therapy over 12 months. We studied event-free survival, hospitalizations, lung function, arterial blood gases (ABG), sleep quality and health-related quality of life.

Effects of exercise and airway clearance (positive expiratory pressure) on mucus clearance in cystic fibrosis: a randomised crossover trial.

Exercise improves mucus clearance in people without lung disease and those with chronic bronchitis. No study has investigated exercise alone for mucus clearance in cystic fibrosis (CF). The aim of this study was to compare the effects of treadmill exercise to resting breathing and airway clearance with positive expiratory pressure (PEP) therapy on mucus clearance in adults with CF.

Management of bronchiectasis in adults.

Once neglected in research and underappreciated in practice, there is renewed interest in bronchiectasis unrelated to cystic fibrosis. Bronchiectasis is a chronic lung disease characterised by chronic cough, sputum production and recurrent pulmonary exacerbations. It is diagnosed radiologically on high resolution computed tomography chest scan by bronchial dilatation (wider than the accompanying artery).

Use of extracorporeal membrane oxygenation in cystic fibrosis in an Australian cystic fibrosis centre.

Extracorporeal membrane oxygenation (ECMO) support is used in selected patients with cystic fibrosis (CF) as a bridge to transplantation. Our aim was to describe briefly treatment and outcomes of six CF patients who received ECMO. One patient received a lung transplant and another recovered from acute respiratory failure.

The use of an alternate side lying positioning strategy during inhalation therapy does not prolong nebulisation time in adults with Cystic Fibrosis: a randomised crossover trial.

Background: Inhalation of nebulised medications is performed in upright sitting to maximise lung volumes. The pattern of deposition is poor for inhaled medications in people with Cystic Fibrosis. The pattern tends to be non-uniform and typically the upper lobes receive a reduced dose compared to the rest of the lung.

Hypoglycaemia in cystic fibrosis: An analysis of a single centre adult cystic fibrosis clinic.

Background: Hypoglycaemia in cystic fibrosis (CF) is known to occur during oral glucose tolerance tests (OGTT) and continuous glucose monitoring, however demographic, clinical and mechanistic data are limited. The aims of this study were to review patient electronic medical records (EMR) in order to 1) describe patient characteristics of a university teaching hospital CF clinic, 2) determine the prevalence of hypoglycaemia on OGTT and explore associations with demographic and clinical characteristics, and 3) explore patient reported symptoms suggestive of hypoglycaemia documented in the EMR.

Methods: Adults who attended the RPA CF clinic between January 2009 to April 2016 were included in the study.

Effects of treadmill exercise versus Flutter® on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial.

Background: Treadmill exercise and airway clearance with the Flutter® device have previously been shown to improve mucus clearance mechanisms in people with cystic fibrosis (CF) but have not been compared. It is therefore not known if treadmill exercise is an adequate form of airway clearance that could replace established airway clearance techniques, such as the Flutter®. The aim of this study was to evaluate respiratory flow, sputum properties and subjective responses of treadmill exercise and Flutter® therapy, compared to resting breathing (control).

Bilateral adrenal haemorrhages: a crucial incidental finding.

Adrenal haemorrhage is a rare condition that has the potential to cause life-threatening adrenal insufficiency, especially if it affects both the adrenal glands. The difficulty in diagnosing adrenal haemorrhages lies in the non-specific clinical presentation including hypotension and abdominal pain. The following case report demonstrates the possible clinical presentations of non-traumatic adrenal haemorrhages and the method of diagnosing and treating adrenal insufficiency.

A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis.

Background: The mucoactive effects of hypertonic saline should promote exacerbation resolution in people with cystic fibrosis (CF).

Objectives: To determine the effects of hypertonic saline inhalation during hospitalisation for exacerbation of CF on length of stay, lung function, symptoms, oxygenation, exercise tolerance, quality of life, bacterial load and time to next hospitalisation.

Methods: 132 adults with an exacerbation of CF were randomised to inhale three nebulised doses a day of either 4 mL 7% saline or a taste-masked control of 0.

Exercise training to improve exercise capacity and quality of life in people with non-malignant dust-related respiratory diseases.

Background: Non-malignant dust-related respiratory diseases, such as asbestosis and silicosis, are similar to other chronic respiratory diseases and may be characterised by breathlessness, reduced exercise capacity and reduced health-related quality of life. Some non-malignant dust-related respiratory diseases are a global health issue and very few treatment options, including pharmacological, are available. Therefore, examining the role of exercise training is particularly important to determine whether exercise training is an effective treatment option in non-malignant dust-related respiratory diseases.

Non-invasive ventilation used as an adjunct to airway clearance treatments improves lung function during an acute exacerbation of cystic fibrosis: a randomised trial.

Question: During an acute exacerbation of cystic fibrosis, is non-invasive ventilation beneficial as an adjunct to the airway clearance regimen?

Design: Randomised controlled trial with concealed allocation and intention-to-treat analysis.

Participants: Forty adults with moderate to severe cystic fibrosis lung disease and who were admitted to hospital for an acute exacerbation.

Intervention: Comprehensive inpatient care (control group) compared to the same care with the addition of non-invasive ventilation during airway clearance treatments from Day 2 of admission until discharge (experimental group).

Physical activity in people with asbestos related pleural disease and dust-related interstitial lung disease: An observational study.

This study aimed to measure the levels of physical activity (PA) in people with dust-related pleural and interstitial lung diseases and to compare these levels of PA to a healthy population. There is limited data on PA in this patient population and no previous studies have compared PA in people with dust-related respiratory diseases to a healthy control group. Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease (ARPD) and a healthy age- and gender-matched population wore the SenseWear(®) Pro3 armband for 9 days.

Exercise training for asbestos-related and other dust-related respiratory diseases: a randomised controlled trial.

Background: The study aimed to determine the short and long-term effects of exercise training on exercise capacity and health-related quality of life (HRQoL) compared to usual care in people with dust-related pleural and interstitial respiratory diseases. No previous studies have specifically evaluated exercise training in this patient population.

Methods: Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease were recruited and randomised to an eight-week exercise training group (EG) or a control group (CG) of usual care.

Modulation of gene expression by Pseudomonas aeruginosa during chronic infection in the adult cystic fibrosis lung.

Chronic Pseudomonas aeruginosa infection is the leading cause of morbidity and mortality in cystic fibrosis (CF) patients. P. aeruginosa isolates undergo significant transcriptomic and proteomic modulation as they adapt to the niche environment of the CF lung and the host defences.

Functional exercise capacity and health-related quality of life in people with asbestos related pleural disease: an observational study.

Background: Functional exercise capacity in people with asbestos related pleural disease (ARPD) is unknown and there are no data on health-related quality of life (HRQoL). The primary aims were to determine whether functional exercise capacity and HRQoL were reduced in people with ARPD. The secondary aim was to determine whether functional exercise capacity was related to peak exercise capacity, HRQoL, physical activity or respiratory function.

Shared Pseudomonas aeruginosa genotypes are common in Australian cystic fibrosis centres.

Recent molecular-typing studies suggest cross-infection as one of the potential acquisition pathways for Pseudomonas aeruginosa in patients with cystic fibrosis (CF). In Australia, there is only limited evidence of unrelated patients sharing indistinguishable P. aeruginosa strains.

Pseudomonas aeruginosa strains from the chronically infected cystic fibrosis lung display increased invasiveness of A549 epithelial cells over time.

The invasive properties of Pseudomonas aeruginosa pose a serious threat to the wellbeing of cystic fibrosis (CF) patients; however the specific factors affecting invasiveness are not well understood, especially in chronic infection. This study characterises the invasive profiles of sequential isolates of the same P. aeruginosa strain collected five to eight years apart from five chronically infected adult CF patients.

Arm exercise training in chronic obstructive pulmonary disease: a randomised controlled trial.

The aim of this study was to compare the effects of arm endurance training, arm strength training, a combination of arm endurance and strength training, and no arm training on endurance arm exercise capacity. A randomised controlled trial was undertaken with chronic obstructive pulmonary disease subjects randomised into one of four groups to complete 8 weeks of training: (a) arm endurance training (endurance group) consisting of supported and unsupported arm exercises, (b) arm strength training (strength group) using weight machines, (c) a combination of arm endurance and arm strength training (combined group), or (d) no arm training (control group). The primary outcome measurement was endurance arm exercise capacity measured by an endurance arm crank test.